The little-known cancer that 30,000 people are living with

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Neuroendocrine tumours fall into the group of less common cancers, but these account for 50 per cent of all cancers – and 50 per cent of all cancer deaths – in the UK. Collectively, neuroendocrine cancers (NETs) are more common than testicular, ovarian, cervical and thyroid cancers, but are much less well-known and more likely to be misdiagnosed.

This ‘constellation of rare cancers’ requires myriad tests before diagnosis can be confirmed and, in my friend’s case, has led to complicated surgery

The neuroendocrine system is formed by a small portion of the brain, called the hypothalamus, linking the nervous system to the endocrine system via the pituitary gland. The system governs and regulates the body temperature, reproduction, metabolism, hunger, thirst, energy utilisation, blood pressure and the release of other hormones within the body. The tumours arise from this system.

The tumours can be benign or malignant and are found, most commonly, in the intestine but can occur in the pancreas, lung and elsewhere in the body. The tumours are treated as a group because they share some common features and treatment pathways.

Late diagnosis is a real problem with NETs and happens because the symptoms – like fatigue, asthma and digestive problems – tend to be attributed to more common conditions like irritable bowel syndrome (a common misdiagnosis in ovarian cancer too), Crohn’s disease, peptic ulcer disease, gastritis, asthma, blood glucose, blood pressure problems or the menopause. Consequently, a NETs diagnosis tends to come when the cancer is at an advanced stage – possibly with a secondary tumour - making effective treatment more difficult.

Some patients have an associated syndrome, the most common of which is carcinoid syndrome, caused by the NETs' cells over-producing a particular hormone, such as serotonin, histamine, somatostatin or chromogranin A. Wheezing, diarrhoea, flushing, abdominal pain, palpitations, high or low blood pressure, or an altered mental state can be signs of this syndrome – which can escalate to a coma and a crisis stage.

Even though an estimated 30,000 people in the UK are living with the disease – and 4,000 more are diagnosed each year – awareness of NETs and its symptoms is in its infancy.

I knew about NETs only because the charity devoted to raising awareness and funding research – the Net Patient Foundation – is a member of Cancer52 , the umbrella organisation that represents 90 small cancer charities, all of whom are working for everyone affected by the rare and less common cancers.

So, when Alan – a friend of mine, already suffering from Parkinson’s disease – was diagnosed with NETs, I was able to point him in the direction of the Net Patient Foundation, where he found a massive amount of information and support.

Wheezing, diarrhoea, flushing, abdominal pain, palpitations, high or low blood pressure, or an altered mental state can be signs of this syndrome

Nikie Jervis, the foundation's patient support manager, told me that, apart from listening, educating, informing, counselling, helping to alleviate pain, fear and uncertainty, one of her important jobs is to ‘interpret the medical jargon’. The foundation lobbies to raise awareness, provides education and information to GPs and sufferers – indeed, the ‘library’ which arrived for Alan covered every possible aspect of NETs – and offers a forum for patients, a support service with a Helpline and Net Natter Groups.

The Foundation celebrated its tenth birthday last year, with a video presented by the comedy writer Andy Hamilton, who explains the initial foundation of the charity by his friend, Liddy Oldroyd – the talented television director who worked on ‘Drop the Dead Donkey’, ‘After Henry’, ‘Spitting Image’ and many other shows.

Liddy was diagnosed with neuroendocrine carcinoid cancer and, before she died in 2002, she and her husband founded ‘Living with Carcinoid’ – a support group for fellow sufferers – as well as raising funds to help finance research at the Royal Free Hospital, where Liddy was treated. From this grew the Net Patient Foundation, launched by Catherine Bouvier, with the help of Andrew Geach and the medical journalist, Cathy Kalamis – both patients.

NETs were relatively unheard of eleven years ago, but through the work of the Net Patient Foundation, awareness and information about the condition is leading to earlier diagnoses and correct treatments. The charity is part of NHS England’s Cancer Strategy and is working with Public Health England to establish the number of people with the condition. Last year the Foundation took their ‘banner’ pens and supportive leaflets to the GPs’ annual conference. Each pen reveals a strip of information about NETs, to remind the GP that the symptoms presented might need to be investigated further.

This ‘constellation of rare cancers’ requires myriad tests before diagnosis can be confirmed and, in my friend’s case, has led to complicated surgery. However, Alan is slowly regaining his strength and – thanks to Nikie and the Net Patient Foundation – has the support and information to help him go forwards with confidence on his cancer journey.